Extract
Pulmonary arterial hypertension (PAH) is a severe progressive disease characterised by obliterative vascular remodelling and increased resistance in small and medium-sized pulmonary arteries (PAs). Contributing factors to PAH pathogenesis include genetic mutations such as those in bone morphogenetic protein receptor type 2 (BMPR2), perivascular inflammation, systemic immune dysregulation, and an imbalance of pulmonary vascular cell proliferation versus apoptosis [1]. PAH remains a malignant condition, despite therapeutic advances, with usually irreversible remodelling of resistance vessels. Morbidity relates to exercise-related breathlessness symptoms, reduced quality of life and, ultimately, increased mortality follows the onset of right ventricular (RV) dysfunction due to uncoupling of the RV–PA unit [2].
Tweetable abstract
Sotatercept may play a crucial role in a reverse remodelling approach for PAH treatment, as supported by the ECHO and haemodynamic data from a post hoc analysis of the STELLAR trial https://bit.ly/3Pd5FRM
Footnotes
Conflict of interest: L.C. Price and C. McCabe have no relevant conflicts of interest related to this work. J. Weatherald reports grants or contracts paid to his institution from AstraZeneca, Bayer, Janssen and Merck, consulting fees and honoraria from Janssen and Merck, payment for expert testimony from Sprigings Intellectual Property Law, travel support from Janssen, participation on a data safety and monitoring board or advisory board for Janssen, Acceleron and the Université de Laval, and has an unpaid leadership role at the Pulmonary Hypertension Association of Canada.
- Received September 6, 2023.
- Accepted September 7, 2023.
- Copyright ©The authors 2023. For reproduction rights and permissions contact permissions{at}ersnet.org
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